In 2016, ESPEN published the guideline for Chronic Intestinal Failure (CIF) in adults. An updated version of ESPEN guidelines on CIF due to benign disease in adults was devised in order to incorporate new evidence since the publication of the previous ESPEN guidelines.

The grading system of the Scottish Intercollegiate Guidelines Network (SIGN) was used to grade the literature. Recommendations were graded according to the levels of evidence available as A (strong), B (conditional), 0 (weak) and Good practice points (GPP). The recommendations of the 2016 guideline (graded using the GRADE system) which were still valid, because no studies supporting an update were retrieved, were reworded and re-graded accordingly.

The recommendations of the 2016 guideline were reviewed, particularly focusing on definitions, and new chapters were included to devise recommendations on IF centers, chronic enterocutaneous fistulas, costs of IF, caring for CIF patients during pregnancy, transition of patients from pediatric to adult centers. The new guideline consist of 149 recommendations and 16 statements which were voted for consensus by ESPEN members, online in July 2022 and at conference during the annual Congress in September 2022. The Grade of recommendation is GPP for 96 (64.4%) of the recommendations, 0 for 29 (19.5%), B for 19 (12.7%), and A for only five (3.4%). The grade of consensus is "strong consensus" for 148 (99.3%) and "consensus" for one (0.7%) recommendation. The grade of consensus for the statements is "strong consensus" for 14 (87.5%) and "consensus" for two (12.5%).

It is confirmed that CIF management requires complex technologies, multidisciplinary and multiprofessional activity, and expertise to care for the underlying gastrointestinal disease and to provide HPN support. Most of the recommendations were graded as GPP, but almost all received a strong consensus.

A high-output stoma (HOS) or fistula is when small bowel output causes water, sodium and often magnesium depletion. This tends to occur when the output is >1.5 -2.0 L/24 hours though varies according to the amount of food/drink taken orally. An HOS occurs in up to 31% of small bowel stomas. A high-output enterocutaneous fistula may, if from the proximal small bowel, behave in the same way and its fluid management will be the same as for an HOS. The clinical assessment consists of excluding causes other than a short bowel and treating them (especially partial or intermittent obstruction). A contrast follow through study gives an approximate measurement of residual small intestinal length (if not known from surgery) and may show the quality of the remaining small bowel. If HOS is due to a short bowel, the first step is to rehydrate the patient so stopping severe thirst. When thirst has resolved and renal function returned to normal, oral hypotonic fluid is restricted and a glucose-saline solution is sipped. Medication to slow transit (loperamide often in high dose) or to reduce secretions (omeprazole for gastric acid) may be helpful. Subcutaneous fluid (usually saline with added magnesium) may be given before intravenous fluids though can take 10-12 hours to infuse. Generally parenteral support is needed when less than 100 cm of functioning jejunum remains. If there is defunctioned bowel in situ, consideration should be given to bringing it back into continuity.

Chronic Intestinal Failure (CIF) is the long-lasting reduction of gut function, below the minimum necessary for the absorption of macronutrients and/or water and electrolytes, such that intravenous supplementation is required to maintain health and/or growth. CIF is the rarest organ failure. Home parenteral nutrition (HPN) is the primary treatment for CIF. No guidelines (GLs) have been developed that address the global management of CIF. These GLs have been devised to generate comprehensive recommendations for safe and effective management of adult patients with CIF.

The GLs were developed by the Home Artificial Nutrition & Chronic Intestinal Failure Special Interest Group of ESPEN. The GRADE system was used for assigning strength of evidence. Recommendations were discussed, submitted to Delphi rounds, and accepted in an online survey of ESPEN members.

The following topics were addressed: management of HPN; parenteral nutrition formulation; intestinal rehabilitation, medical therapies, and non-transplant surgery, for short bowel syndrome, chronic intestinal pseudo-obstruction, and radiation enteritis; intestinal transplantation; prevention/treatment of CVC-related infection, CVC-related occlusion/thrombosis; intestinal failure-associated liver disease, gallbladder sludge and stones, renal failure and metabolic bone disease. Literature search provided 623 full papers. Only 12% were controlled studies or meta-analyses. A total of 112 recommendations are given: grade of evidence, very low for 51%, low for 39%, moderate for 8%, and high for 2%; strength of recommendation: strong for 63%, weak for 37%.

CIF management requires complex technologies, multidisciplinary and multiprofessional activity, and expertise to care for both the underlying gastrointestinal disease and to provide HPN support. The rarity of the condition impairs the development of RCTs. As a consequence, most of the recommendations have a low or very low grade of evidence. However, two-thirds of the recommendations are considered strong. Specialized management and organization underpin these recommendations.

Undernutrition as well as specific nutrient deficiencies has been described in patients with Crohn's disease (CD), ulcerative colitis (UC) and short bowel syndrome. In the latter, water and electrolytes disturbances may be a major problem. The present guidelines provide evidence-based recommendations for the indications, application and type of parenteral formula to be used in acute and chronic phases of illness. Parenteral nutrition is not recommended as a primary treatment in CD and UC. The use of parenteral nutrition is however reliable when oral/enteral feeding is not possible. There is a lack of data supporting specific nutrients in these conditions. Parenteral nutrition is mandatory in case of intestinal failure, at least in the acute period. In patients with short bowel, specific attention should be paid to water and electrolyte supplementation. Currently, the use of growth hormone, glutamine and GLP-2 cannot be recommended in patients with short bowel.

Short bowel syndrome is a chronic malabsorptive state usually resulting from extensive small bowel resections. A combination of diarrhea, nutrient malabsorption, dysmotility, and bowel dilatation may constitute the clinical symptomatology of this syndrome. The remaining bowel undergoes a process called adaptation, which may replace lost intestinal function. Chronic complications include nutrient, electrolyte, and vitamin deficiencies. Therapy depends largely on the administration of various factors stimulating intestinal adaptation of the remaining bowel. If the patient despite medical therapy fails to return to oral diet alone, then long-term parenteral nutrition is required. However, long-term parenteral nutrition may gradually induce cholestatic liver disease. Surgical methods may be required for treatment including intestinal transplantation, as a last resort for the treatment of end-stage intestinal failure. The goal of this review is to analyze the clinical spectrum and pathophysiologic aspects of the syndrome, the process of intestinal adaptation, and to outline the medical and surgical methods currently used to treat this complicated group of patients.

A new definition of intestinal failure is of reduced intestinal absorption so that macronutrient and/or water and electrolyte supplements are needed to maintain health or growth. Severe intestinal failure is when parenteral nutrition and/or fluid are needed and mild intestinal failure is when oral supplements or dietary modification suffice. Treatment aims to reduce the severity of intestinal failure. In the peri-operative period avoiding the administration of excessive amounts of intravenous saline (9 g NaCl/l) may prevent a prolonged ileus. Patients with intermittent bowel obstruction may be managed with a liquid or low-residue diet. Patients with a distal bowel enterocutaneous fistula may be managed with an enteral feed absorbed by the proximal small bowel while no oral intake may be needed for a proximal bowel enterocutaneous fistula. Patients undergoing high-dose chemotherapy can usually tolerate jejunal feeding. Rotating antibiotic courses may reduce small bowel bacterial overgrowth in patients with chronic intestinal pseudoobstruction. Restricting oral hypotonic fluids, sipping a glucose-saline solution (Na concentration of 90-120 mmol/l) and taking anti-diarrhoeal or anti-secretory drugs, reduces the high output from a jejunostomy. This treatment allows most patients with a jejunostomy and > 1 m functioning jejunum remaining to manage without parenteral support. Patients with a short bowel and a colon should consume a diet high in polysaccharides, as these compounds are fermented in the colon, and low in oxalate, as 25% of the oxalate will develop as calcium oxalate renal stones. Growth factors normally produced by the colon (e.g. glucagon-like peptide-2) to induce structural jejunal adaptation have been given in high doses to patients with a jejunostomy and do marginally increase the daily energy absorption.

Short bowel syndrome most commonly results after bowel resections for Crohn's disease. The normal human small intestinal length ranges from about 3 to 8 m, thus if the initial small intestinal length is short, a relatively small resection of the intestine may result in the problems of a short bowel. Two types of patient with a short bowel are encountered in clinical practice: those with their jejunum anastomosed to a functioning colon, and those with a jejunostomy. Both types of patient have problems absorbing adequate macronutrients, and both need long-term vitamin B12 therapy. Patients with a jejunostomy also have major problems with large stomal losses of water, sodium, and magnesium. This high-volume jejunostomy output is treated by restricting oral fluids, giving a glucose-saline solution to drink, and using drugs that either reduce gastrointestinal motility (loperamide or codeine phosphate) or secretions (H2 antagonists, proton pump inhibitors, or octreotide). Patients whose jejunal length is less than 100 cm, and whose stomal output is greater than their oral intake, benefit most from antisecretory drugs. In patients with a retained colon, bacterial fermentation of unabsorbed carbohydrate in the colon results in energy being salvaged. However, they have increased oxalate absorption and a 25% chance of developing calcium oxalate renal stones. Thus patients with a colon are advised to eat a high-energy diet rich in carbohydrate but low in oxalate. Patients with a jejunostomy need a high-energy iso-osmolar diet with added salt. Both patient types have a 45% prevalence of gallstones. With current therapy most patients with a short bowel have a normal body mass index and a good quality of life.

A shortened small intestine may end at a stoma or be anastomosed to the colon. Patients with a jejunostomy, but not those with a colon, lose large amounts of sodium. The intake and absorption of sodium can be increased by sipping a sodium-glucose solution; stomal loss can be reduced by restricting water or low-sodium drinks. If a stoma is situated less than 100 cm along the jejunum, a constant negative sodium balance may necessitate parenteral saline supplements. Gastric anti-secretory drugs or a somatostatin analogue reduce jejunostomy losses in such patients but do not restore a positive sodium balance. Loperamide or codeine phosphate benefit some patients. Magnesium deficiency can usually be corrected by oral magnesium oxide supplements. An elemental or hydrolysed diet is not beneficial. Patients with a jejunostomy can maintain a normal diet without fat reduction. When the colon is present, unabsorbed carbohydrate is fermented to absorbable short chain fatty acids. Unabsorbed long chain fatty acids and bile salts cause watery diarrhoea and increased colonic oxalate absorption with hyperoxaluria. Such patients benefit from a high carbohydrate, low-fat and low-oxalate diet. Parenteral nutrition is needed only by the few patients unable to maintain health or avoid socially disabling diarrhoea despite these measures.

The effect of 25-hydroxyvitamin D (25OHD), given orally during the reversal of hypomagnesaemia, was studied in five patients with hypomagnesaemic hypocalcaemia and low serum levels of 25OHD and 1,25-dihydroxyvitamin D (1,25(OH)2D). The results were compared to those obtained in five other patients with similar initial levels of magnesium, calcium, 25OHD and 1,25(OH)2D who did not receive 25OHD. Serum levels of 1,25(OH)2D in the ten hypomagnesaemic patients were lower than in ten control subjects with low serum levels of 25OHD. The reversal of hypomagnesaemia was similar in the two groups of patients and elicited a similar increase of circulating iPTH levels. The expected increase of circulating 25OHD was observed in patients supplemented with 25OHD; their circulating 1,25(OH)2D rose within 48 h to normal levels, contrasting with the delayed and poor increase of 1,25(OH)2D in patients receiving no 25OHD. The evolution of serum calcium was however identical in the two groups. Our results suggest that vitamin D deficiency was a significant factor leading to low circulating levels of 1,25(OH)2D in hypomagnesaemic hypocalcaemic patients. The biological consequences of low serum 1,25(OH)2D in these patients remain unclear, but clearly, normal levels of 1,25(OH)2D are not essential for the correction of hypomagnesaemic hypocalcaemia.

Osteoporosis primarily affects women and is a bone disease of great social, medical and economic importance. The pathogenesis is thought to be of multifactorial origin. The current work discusses nutritional aspects relative to osteoporosis. In this aspect, calcium supply seems to have the largest preventional importance. In contrast, food statistics and individual food records show that calcium intake of females is below the DGE's recommendation of 800 mg/daily. Furthermore, in view of skeletal integrity, calcium intake should be increased up to 1,500 mg/daily after menopause, because of endocrinological changes in calcium metabolism. Therefore, calcium intake on the whole is usually found to not be sufficient. The importance of further nutritional aspects for manifestation of osteoporosis are discussed. In some cases, influences on skeletal integrity are quite evident, whereas the literature has only infrequently drawn conclusions about the pathogenesis of osteoporosis.

Significant advances have been made in the last 30 years in the understanding of Ca, PO4, and Mg homeostasis in ruminants. Despite these advances, the primary cellular or molecular lesions responsible for failure of homeostasis have yet to be identified. Suggested causes, such as primary hormone deficiencies, have been eliminated, and we now believe that aging and nutrition can reduce the ability of intestine, bone, and kidney to respond rapidly to the hormone signals responsible for homeostasis during rapid increases in demand for these minerals. Further research is required before these lesions can be identified and new knowledge applied to the development of economical, effective programs that prevent milk fever and hypomagnesemic tetany. Until then, diseases such as milk fever will continue to affect 8 to 9 per cent of our dairy cows, and dairy farmers will continue to spend millions of dollars a year for treatment of the primary disease and the many secondary problems that result from these diseases.